Primary CNS Posttransplant Lymphoproliferative Disease (PTLD): An International Report of 84 Cases in the Modern Era. Issue 6 (30th May 2013)

• Record Type: Journal Article
• Authors: Evens, A. M.; Choquet, S.; Kroll‐Desrosiers, A. R.; Jagadeesh, D.; Smith, S. M.; Morschhauser, F.; Leblond, V.; Roy, R.; Barton, B.; Gordon, L. I.; Gandhi, M. K.; Dierickx, D.; Schiff, D.; Habermann, T. M.; Trappe, R.
• Is Part Of: American journal of transplantation. Volume 13:Issue 6(2013:Jun.)
• Page Start: 1512
• Page End: 1522
• Publication Date: 2013-05-30
• Subjects: Transplantation of organs, tissues, etc -- Periodicals; 617.95
• DOI: 10.1111/ajt.12211
• Languages: English
• ISSNs: 1600-6135

Abstract

<abstract abstract-type="main" xml:lang="en"> <title>Abstract</title> <sec id="ajt12211-sec-0001" sec-type="section"> <p>We performed a multicenter, International analysis of solid organ transplant (SOT)‐related primary central nervous system (PCNS) posttransplant lymphoproliferative disease (PTLD). Among 84 PCNS PTLD patients, median time of SOT‐to‐PTLD was 54 months, 79% had kidney SOT, histology was monomorphic in 83% and tumor was EBV+ in 94%. Further, 33% had deep brain involvement, 10% had CSF involvement, while none had ocular disease. Immunosuppression was reduced in 93%; additional first‐line therapy included high‐dose methotrexate (48%), high‐dose cytarabine (33%), brain radiation (24%) and/or rituximab (44%). The overall response rate was 60%, while treatment‐related mortality was 13%. With 42‐month median follow‐up, three‐year progression‐free survival (PFS) and overall survival (OS) were 32% and 43%, respectively. There was a trend on univariable analysis for improved PFS for patients who received rituximab and/or high‐dose cytarabine. On multivariable Cox regression, poor performance status predicted inferior PFS (HR 2.61, 95% CI 1.32–5.17, p = 0.006), while increased LDH portended inferior OS (HR 4.16, 95% CI 1.29–13.46, p = 0.02). Moreover, lack of response to first‐line therapy was the most dominant prognostic factor on multivariable analysis (HR 8.70, 95% CI 2.56–29.57, p = 0.0005). Altogether, PCNS PTLD appears to represent a distinct clinicopathologic entity within the PTLD spectrum that is associated with renal SOT, occurs late, is monomorphic and retains EBV positivity.</p> </sec> </abstract>